title: Acute promyelocytic leukemia following chemotherapy for EBV-associated hemophagocytic lymphohistiocytosis.
abstract: We report a case of chemotherapy-related acute promyelocytic leukemia (APL) following therapy with VP-16/etoposide for EBV-associated hemophagocytic lymphohistiocytosis (HLH). A 17-month-old male presented with fever and lymphadenopathy. Bone marrow and liver biopsies showed hemophagocytosis. He responded well to chemotherapy including dexamethasone, VP-16/etoposide, and cyclosporine. One and a half year later, he developed fever and pancytopenia. Clinical work-up revealed APL with t(15;17)(q22;q12);PML-RARα translocation. He underwent chemotherapy for APL and is in remission 8 years after diagnosis. Alternative non-leukemogenic agents to effectively treat HLH would be desirable.
authors.affiliation: Department of Pathology, Georgetown University Hospital, Washington, District of Columbia 20007, USA.
authors.forename: Srividya
authors.initials: S
authors.lastname: Sathiyamoorthy
authors.forename: Aziza
authors.initials: A
authors.lastname: Shad
authors.forename: Metin
authors.initials: M
authors.lastname: Ozdemirli
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000009:adverse effects*; D002884:Chromosomes, Human, Pair 15 / Q000235:genetics; D002886:Chromosomes, Human, Pair 17 / Q000235:genetics; D016572:Cyclosporine / Q000008:administration & dosage; D003907:Dexamethasone / Q000008:administration & dosage; D020031:Epstein-Barr Virus Infections / Q000150:complications* / Q000188:drug therapy* / Q000821:virology; D005047:Etoposide / Q000008:administration & dosage; D004854:Herpesvirus 4, Human / Q000472:pathogenicity; D006801:Humans; D017404:In Situ Hybridization, Fluorescence; D007223:Infant; D007621:Karyotyping; D015473:Leukemia, Promyelocytic, Acute / Q000139:chemically induced* / Q000175:diagnosis / Q000188:drug therapy; D008206:Lymphatic Diseases; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications* / Q000188:drug therapy* / Q000821:virology; D008297:Male; D015514:Oncogene Proteins, Fusion / Q000235:genetics; D014178:Translocation, Genetic / Q000235:genetics
chemical_list: D015514:Oncogene Proteins, Fusion; C093476:promyelocytic leukemia-retinoic acid receptor alpha fusion oncoprotein; D005047:Etoposide; D003907:Dexamethasone; D016572:Cyclosporine